Classification of aplasia cutis congenita

Category	Body area affected	Associated abnormalities	Inheritance
Group 1:
Scalp ACC without multiple anomalies	Scalp, usually vertex	Cleft lip and palate; tracheoesophageal fistula; double cervix and uterus; patent ductus arteriosus; omphalocele; polycystic kidney; mental retardation; cutis marmorata telangiectatica congenita	Autosomal dominant or sporadic
Group 2:
Scalp ACC with associated limb abnormalities	Midline scalp	Limb reduction abnormalities; two to three syndactyly; clubfoot; nail absence or dystrophy; skin tags on toes; persistent cutis marmorata; encephalocele; woolly hair; hemangioma; heart disease; cryptorchidism; postaxial polydactyly (one family)	Autosomal dominant
Group 3:
Scalp ACC with associated epidermal and organoid nevi	Scalp, may be asymmetric	Corneal opacities; scleral dermoids; eyelid colobomas; psychomotor retardation; seizures	Sporadic
Group 4:
ACC overlying embryologic malformations	Abdomen, lumbar skin, scalp; any site	Meningomyeloceles; spinal dysraphia; cranial stenosis; congenital midline porencephaly; leptomeningeal angiomatosis; ectopia of ear; omphalocele; gastroschisis	Depends on underlying condition
Group 5:
ACC with associated fetus papyraceus or placental infarcts	Multiple, symmetric areas, often stellate or linear, on scalp, chest, flanks, axillae, and extremities	Single umbilical artery; developmental delay; spastic paralysis; nail dystrophy; clubbed hands and feet; amniotic bands	Sporadic
Group 6: ACC associated with EB:
Blistering, usually localized, without multiple congenital anomalies	Extremities	Blistering of skin and/or mucous membranes; absent or deformed nails; metatarsus varus; congenital absence of kidney (seen in cases of recessive, dystrophic EB; dominant, dystrophic EB; and EB simplex)	Depends on EB type: may be autosomal dominant or recessive
Widespread skin fragility with congenital anomalies	Large areas on extremities and torso	Pyloric or duodenal atresia; abnormal ears and nose; ureteral stenosis; renal abnormalities; arthrogryposis; amniotic bands; nail dystrophy	Autosomal recessive
Group 7:
ACC localized to extremities without blistering	Pretibial areas; dorsal aspects of hands and feet; extensor areas of wrists	None	Autosomal dominant or recessive
Group 8:
ACC caused by specific teratogens	Scalp (with methimazole); any area (with varicella and herpes simplex infections)	Imperforate anus (methimazole); signs of intrauterine infection with varicella and herpes simplex infections	Not inherited
Group 9:
ACC associated with malformation syndromes	Scalp; any location	Trisomy 13; 4p deletion syndrome; many ectodermal dysplasias; Johanson-Blizzard syndrome; focal dermal hypoplasia; amniotic band disruption complex; XY gonadal dysgenesis	Varies, depending on specific syndrome

ACC: aplasia cutis congenita; EB: epidermolysis bullosa.

Reproduced from: Frieden IJ. Aplasia cutis congenital: A clinical review and proposal for classification. J Am Acad Dermatol 1986; 14:646. Table used with the permission of Elsevier Inc. All rights reserved.

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Classification of aplasia cutis congenita